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Mouse Anti-DHH Recombinant Antibody (1B12) (CBMAB-D0827-YC)


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Summary

Host Animal
Mouse
Specificity
Human
Clone
1B12
Antibody Isotype
IgG2a, κ
Application
ELISA, WB

Basic Information

Immunogen
DHH (NP_066382, 210 a.a. ~ 310 a.a) partial recombinant protein with GST tag. The immunogen sequence: SGERKGLREL HRGDWVLAAD ASGRVVPTPV LLFLDRDLQR RASFVAVETE WPPRKLLLTP WHLVFAARGP APAPGDFAPV FARRLRAGDS VLAPGGDALR P*
Specificity
Human
Antibody Isotype
IgG2a, κ
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Storage
Store at 4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name
desert hedgehog homolog (Drosophila)
Introduction
DHH belongs to the hedgehog family. The hedgehog gene family encodes signaling molecules that play an important role in regulating morphogenesis. This protein is predicted to be made as a precursor that is autocatalytically cleaved; the N-terminal portion is soluble and contains the signalling activity while the C-terminal portion is involved in precursor processing. More importantly, the C-terminal product covalently attaches a cholesterol moiety to the N-terminal product, restricting the N-terminal product to the cell surface and preventing it from freely diffusing throughout the organism. Defects in this protein have been associated with partial gonadal dysgenesis (PGD) accompanied by minifascicular polyneuropathy. This protein may be involved in both male gonadal differentiation and perineurial development.
Entrez Gene ID
50846
UniProt ID
O43323
Alternative Names
Desert Hedgehog; HHG-3; Desert Hedgehog (Drosophila) Homolog; Desert Hedgehog Protein; GDXYM; SRXY7;
Function
Intercellular signal essential for a variety of patterning events during development. May function as a spermatocyte survival factor in the testes. Essential for testes development.
Biological Process
Cell-cell signaling Source: InterPro
Cell fate specification Source: GO_Central
Leydig cell differentiation Source: Ensembl
Male sex determination Source: Ensembl
Myelination Source: Ensembl
Osteoblast differentiation Source: Ensembl
Protein autoprocessing Source: InterPro
Regulation of gene expression Source: GO_Central
Regulation of steroid biosynthetic process Source: Ensembl
Response to estradiol Source: Ensembl
Response to estrogen Source: Ensembl
Smoothened signaling pathway Source: GO_Central
Spermatid development Source: Ensembl
Cellular Location
Desert hedgehog protein N-product: Cell membrane. The N-terminal peptide remains associated with the cell surface.
Desert hedgehog protein C-product: Extracellular space. The C-terminal peptide diffuses from the cell.
Involvement in disease
Partial gonadal dysgenesis with minifascicular neuropathy 46,XY (PGD):
Characterized by the presence of a testis on one side and a streak or an absent gonad at the other, persistence of Muellerian duct structures, and a variable degree of genital ambiguity.
46,XY sex reversal 7 (SRXY7):
A disorder of sex development. Affected individuals have a 46,XY karyotype but present as phenotypically normal females. SRXY7 patients have no functional gonads.
PTM
The C-terminal domain displays an autoproteolysis activity and a cholesterol transferase activity. Both activities result in the cleavage of the full-length protein and covalent attachment of a cholesterol moiety to the C-terminal of the newly generated N-terminal fragment (N-product). This covalent modification appears to play an essential role in restricting the spatial distribution of the protein activity to the cell surface. The N-product is the active species in both local and long-range signaling, whereas the C-product has no signaling activity (By similarity).
More Infomation

Chapouly, C., Hollier, P. L., Guimbal, S., Cornuault, L., Gadeau, A. P., & Renault, M. A. (2020). Desert hedgehog-driven endothelium integrity is enhanced by gas1 (growth arrest-specific 1) but negatively regulated by Cdon (cell adhesion molecule-related/downregulated by oncogenes). Arteriosclerosis, Thrombosis, and Vascular Biology, 40(12), e336-e349.

Moreau, N., & Boucher, Y. (2020). Hedging against neuropathic pain: role of hedgehog signaling in pathological nerve healing. International Journal of Molecular Sciences, 21(23), 9115.

Pession, A., Lonetti, A., Bertuccio, S., Locatelli, F., & Masetti, R. (2019). Targeting Hedgehog pathway in pediatric acute myeloid leukemia: challenges and opportunities. Expert Opinion on Therapeutic Targets, 23(2), 87-91.

Sasai, N., Toriyama, M., & Kondo, T. (2019). Hedgehog signal and genetic disorders. Frontiers in genetics, 1103.

Abramyan, J. (2019). Hedgehog signaling and embryonic craniofacial disorders. Journal of developmental biology, 7(2), 9.

Skoda, A. M., Simovic, D., Karin, V., Kardum, V., Vranic, S., & Serman, L. (2018). The role of the Hedgehog signaling pathway in cancer: A comprehensive review. Bosnian journal of basic medical sciences, 18(1), 8.

Riaz, S. K., Khan, J. S., Shah, S. T. A., Wang, F., Ye, L., Jiang, W. G., & Malik, M. F. A. (2018). Involvement of hedgehog pathway in early onset, aggressive molecular subtypes and metastatic potential of breast cancer. Cell Communication and Signaling, 16(1), 1-12.

Fattahi, S., Pilehchian Langroudi, M., & Akhavan‐Niaki, H. (2018). Hedgehog signaling pathway: Epigenetic regulation and role in disease and cancer development. Journal of Cellular Physiology, 233(8), 5726-5735.

Akyala, A. I., & Peppelenbosch, M. P. (2018). Gastric cancer and Hedgehog signaling pathway: emerging new paradigms. Genes & cancer, 9(1-2), 1.

Jäger, T., Ocker, M., Kiesslich, T., Neureiter, E., & Neureiter, D. (2017). Thoughts on investigational hedgehog pathway inhibitors for the treatment of cancer. Expert Opinion on Investigational Drugs, 26(2), 133-136.

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For research use only. Not intended for any clinical use.

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